Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: A combined retrospective and case-control study

Gerhard Paul Diller, Konstantinos Dimopoulos, Craig S. Broberg, Mehmet G. Kaya, Utpal Singh Naghotra, Anselm Uebing, Carl Harries, Omer Goktekin, J. Simon R. Gibbs, Michael A. Gatzoulis

Research output: Contribution to journalArticlepeer-review

254 Scopus citations


Aims: To characterize contemporary Eisenmenger patients at a large centre for adult congenital heart disease, assess survival prospects, and identify predictors of death in this population. Methods and results: All Eisenmenger patients under follow-up at our centre since 2000 (n=171, mean age 37±12 years) were included. To identify predictors of mortality, a case-control study was performed. Data including symptoms, functional class, medication, laboratory, and electrocardiographic and echocardiographic parameters are presented. Iron deficiency was common and strongly related to phlebotomy (relative risk 4.1, P<0.0001). Haemoglobin concentration was inversely related to arterial oxygen saturations in iron-replete patients (P<0.001) but not in iron-deficient patients. During a median follow-up of 67 months, 20 patients died. Survival at 40, 50, and 60 years of age was 94, 74, and 52%, respectively. When compared with healthy individuals, median survival was reduced by ∼20 years in Eisenmenger patients and was worst in those with complex lesions. Predictors of mortality included functional class, signs of heart failure, history of clinical arrhythmia, QRS duration and QTc interval, and low serum albumin and potassium levels. Conclusion: Despite good short-term prognosis, life expectancy is markedly reduced in Eisenmenger patients. Markers of heart failure and parameters associated with arrhythmia are of prognostic value in terms of mortality and may guide clinicians caring for Eisenmenger patients.

Original languageEnglish (US)
Pages (from-to)1737-1742
Number of pages6
JournalEuropean heart journal
Issue number14
StatePublished - Jul 2006
Externally publishedYes


  • Congenital heart disease
  • Eisenmenger syndrome
  • Pulmonary hypertension
  • Survival

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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